Pancreatoblastoma
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Pancreatoblastoma is a rare malignant tumour of the pancreas that is predominantly afflicts children.
General
- Very rare.
- Tumour of childhood - age of diagnosis ~5 years old.[1]
- Prognosis ~80% year survival in children[2] more aggressive in adults.
- May be seen in adults.[3]
Associations:[4]
Microscopic
- Acinar-like structures.
- Squamoid corpuscles.
- Undifferentiated component.
DDx:
- Other small round cell tumours.
Image
IHC
Features:[5]
- CK7 +ve - acinar, undifferentiated component.
- CK8 +ve - squamous component.
- CK18 +ve - squamous component.
- CK19 +ve - squamous component.
See also
References
- ↑ Glick, RD.; Pashankar, FD.; Pappo, A.; Laquaglia, MP. (May 2012). "Management of pancreatoblastoma in children and young adults.". J Pediatr Hematol Oncol 34 Suppl 2: S47-50. doi:10.1097/MPH.0b013e31824e3839. PMID 22525406.
- ↑ Bien, E.; Godzinski, J.; Dall'igna, P.; Defachelles, AS.; Stachowicz-Stencel, T.; Orbach, D.; Bisogno, G.; Cecchetto, G. et al. (Oct 2011). "Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT).". Eur J Cancer 47 (15): 2347-52. doi:10.1016/j.ejca.2011.05.022. PMID 21696948.
- ↑ Balasundaram, C.; Luthra, M.; Chavalidthamrong, D.; Chow, J.; Khan, H.; Endres, PJ. (May 2012). "Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology.". JOP 13 (3): 301-3. PMID 22572137.
- ↑ 4.0 4.1 Saif, MW. (2007). "Pancreatoblastoma.". JOP 8 (1): 55-63. PMID 17228135.
- ↑ 5.0 5.1 Nishimata, S.; Kato, K.; Tanaka, M.; Ijiri, R.; Toyoda, Y.; Kigasawa, H.; Ohama, Y.; Nakatani, Y. et al. (Jun 2005). "Expression pattern of keratin subclasses in pancreatoblastoma with special emphasis on squamoid corpuscles.". Pathol Int 55 (6): 297-302. doi:10.1111/j.1440-1827.2005.01829.x. PMID 15943785.